Simultaneous stenting of tightly stenosed patent ductus arteriosus and pulmonary artery bifurcation using two stents (Y stenting): an innovative technique.

Stenting of patent ductus arteriosus (PDA) is a palliative technique that is evolving as an alternative to shunt surgery. Patients with duct-dependant pulmonary circulation and branch pulmonary artery stenosis are often palliated by shunt surgery with repair of branch pulmonary arteries under cardiopulmonary bypass. We present here an 8-month-old male child with duct-dependant pulmonary circulation with bifurcation stenosis who was palliated successfully by transcatheter means. He had stenosed PDA with tight pulmonary artery bifurcation stenosis and underwent successful "Y" stenting of PDA with simultaneous deployment of two stents. He successfully underwent bidirectional Glenn surgery 8 months after the procedure. Simultaneous stenting of bifurcation stenosis of branch pulmonary arteries with two stents has not been described in the literature.

Role of CT angiography in the diagnosis and treatment of popliteal vascular entrapment syndrome.

OBJECTIVE: Popliteal vascular entrapment syndrome is an uncommon congenital abnormality of the anatomic relations between the popliteal vessels and the neighboring musculotendinous structures. The purpose of this essay is to describe the CT angiographic findings in the diagnosis and treatment of this syndrome. CONCLUSION: Digital subtraction angiography is of limited value in the evaluation of popliteal vascular entrapment syndrome and has been replaced by noninvasive imaging techniques, such as Doppler sonography, CT angiography, MRI, and MR angiography.

CT angiography and MRI in patients with popliteal artery entrapment syndrome.

OBJECTIVE: Popliteal artery entrapment syndrome (PAES) is an uncommon congenital anomaly affecting young adults who present with symptoms of calf claudication. It is characterized by various anomalous anatomic relationships between the muscle and arteries in the popliteal fossa, resulting in extrinsic arterial compression. The purpose of this study was to evaluate the potential role of CT angiography (CTA) and MRI in the diagnosis of PAES. MATERIALS AND METHODS: Eight patients (11 legs) who underwent surgical treatment for PAES over a 4-year period were included in this study. All of the cases showed various anomalous relationships between the popliteal artery and the neighboring muscular structures. CTA and MRI showed the detailed anatomy of the region well enough to reveal the cause of arterial entrapment. CTA defined the location and length of the occluded segment and collateral circulation. RESULTS: Characterization and classification based on CTA and MRI findings were consistent with intraoperative photography. CONCLUSION: CTA and MRI can show anatomic variations in the popliteal fossa and may be valuable in the diagnosis of PAES in young adults presenting with intermittent claudication.

The Use of Low-Dose Recombinant Tissue Plasminogen Activator to Treat a Preterm Infant with an Intrauterine Spontaneous Arterial Thromboembolism.

Neonatal thromboembolic events are rare, and only a few cases of intrauterine spontaneous arterial thromboembolisms have been reported in the literature. Thrombolytic therapy with recombinant tissue plasminogen activator is usually the preferred treatment because it has a short half-life, fewer systemic side effects, and a strong, specific affinity for fibrin. Protocols vary from center to center, but there is still no consensus regarding the proper dosage or treatment duration. Herein, we present the case of an intrauterine spontaneous arterial thromboembolism in a preterm infant that completely resolved after being treated with low-dose recombinant tissue plasminogen activator (0.02 mg/kg/h).

Comparison of three-dimensional contrast-enhanced magnetic resonance angiography and axial radiographic angiography for diagnosing congenital stenoses in small pulmonary arteries.

Accuracy of 3-dimensional contrast-enhanced magnetic resonance angiography (MRA) in diagnosing morphology of the branch pulmonary artery (PA) was evaluated in 73 patients (aged 7.2 +/- 6.4 years [mean +/- SD]) with various congenital heart diseases. The presence or absence of localized stenosis of branch PAs, PA diameter, and Nakata's PA index were determined on MRA and axial radiographic angiography, and the results were compared. Sensitivity, specificity, and overall accuracy in detecting branch PA stenoses were 92.7%, 96.2%, and 95.2%, respectively. Correlations between axial radiographic angiography and MRA were excellent in measuring PA diameter (r = 0.956, SEE = 1.49 mm, n = 139) as well as PA index (r = 0.839, SEE = 48.9, n = 37); both p < 0.0001. Bland-Altman plots showed a mean difference +/- SD for PA diameter of 0.17 +/- 1.51 mm and for PA index of 8.5 +/- 50.1. When the main right and left PAs were taken as the first generation, the most distal branches visible on MRA were the 4.7 +/- 0.7 generation with breath-holding (n = 23) and the 3.7 +/- 0.5 without breath-holding (n = 50), respectively (p < 0.0001). Both intra- and interobserver variabilities of MRA measurements were few (9.5 +/- 11.6% and 13.5 +/- 15.0%, respectively, n = 139). In conclusion, 3-dimensional contrast-enhanced MRA enables us to document branch PA morphology clearly in infants and adult patients with congenital cardiovascular defects.

Congenital stenotic arteriopathy with medial dysplasia.

An unusual, uniformly stenotic arteriopathy involving the aorta and its major branches, the pulmonary trunk, and the left and right pulmonary arteries was found in association with a persistent common atrioventricular canal in an otherwise normal stillborn female infant. The uniform arterial thickening was due to hyperplastic medial elastic laminae, which were in an orderly arrangement in the inner two thirds but dysplastic in the outer third of the media of the arteries.

Surgical repair of severe bilateral branch pulmonary artery stenosis.

Management options for severe, bilateral branch pulmonary artery stenosis include percutaneous balloon dilation and direct surgical correction. Results with both balloon angioplasty and operation have been somewhat unpredictable. We report a case of staged surgical correction involving bilateral branch pulmonary artery reconstruction.

Bulboventricular foramen resection: hemodynamic and electrophysiologic results.

BACKGROUND: The two major surgical approaches to the relief of bulboventricular foramen (BVF) obstruction in patients with single left ventricle (LV) are the Damus-Kaye-Stansel (DKS) procedure or direct BVF resection. Theoretical advantages of the DKS include better out-flow gradient relief, lower potential incidences of postoperative heart block and lower incidences of reoperation. Potential disadvantages of this approach include increased semilunar valvar insufficiency, lack of feasibility when attempting septation-type operations for univentricular hearts, and a technically more difficult operation. We report the results of direct surgical BVF resection. METHODS: From June 1990 to June 1999, 9 patients had direct BVF resection performed at our institution. The median age at surgery was 16.5 years (range 1 month to 27 years). Diagnoses in these patients were [S,L,L] single LV (n = 8) and [S,D,D] single LV tricuspid atresia (n = 1). Eight of 9 patients had pulmonary artery bands placed either before BVF resection or at the same time as this procedure. Three patients required reoperation for reobstruction at the BVF (12 total operations in 9 patients). RESULTS: Median preoperative peak systolic gradient across the BVF measured at cardiac catheterization was 47 mm Hg (range 10 to 63 mm Hg). The median peak gradient measured by Doppler echocardiography was 44 mm Hg (range 5 to 125 mm Hg). Eight of 9 patients survived the operation to discharge from the hospital and 7 of 9 are alive at follow-up. At a median follow-up of 22 months (range 5 to 76 months), 8 of 8 surviving patients had an unobstructed BVF as determined by qualitative two-dimensional echocardiography and Doppler color flow imaging. There was one perioperative and one late death 5 months postoperatively (secondary to fungal sepsis). No patient developed new or worsened aortic insufficiency after BVF resection. Eight of 9 patients had no change in AV nodal conduction after surgery. One patient developed Mobitz II heart block requiring postoperative implantation of a pacemaker. CONCLUSIONS: Direct resection of an obstructive BVF can be performed with total relief of obstruction although reoperation may be required. Atrioventricular nodal function can be preserved in most patients with this operative approach, including those with [S,L,L] segmental anatomy.

Developmental occlusive disease of the abdominal aorta and the splanchnic and renal arteries.

Developmental occlusive disease of the abdominal aorta and the renal and splanchnic arteries represent an unusual vascular condition. When unrecognized or untreated this disease is associated with premature death, usually from severe secondary hypertension as a consequence of renovascular stenotic lesions. Strong circumstantial evidence indicates that developmental abnormalities occurring during the fetal union of the two dorsal aortae account for most of the occlusive lesions affecting the abdominal aorta and its visceral branches in these patients. Complete arteriographic studies are necessary to confirm and accurately delineate the disease process. Surgical treatment, which often encompasses complex vascular reconstructive efforts, affords excellent results when carefully planned and executed.

Immediate post-operative balloon dilatation of neonatal long segment stenosis of the descending thoracic aorta.

During surgical repair of a neonatal coarctation it was recognized that this was a rare, previously undescribed form of stenosis of the entire descending thoracic aorta. A few hours after the end-to-end surgical repair, the child underwent successful balloon angioplasty, involving the entire descending thoracic aorta, and in which we intentionally avoided the surgical site.

Idiopathic arterial calcification of infancy in newborn siblings with unusual light and electron microscopic manifestations.

We report two cases of idiopathic arterial calcification of infancy (IACI) occurring in newborn siblings. Unusual gross and light microscopic findings included calcification of multiple nonarterial tissues, renal infarction, and macroscopic aortic involvement. One case manifested an extensive acute panarteritis suggesting that IACI may be the result of an inflammatory or infectious process. Ultrastructural examination confirmed that the mineral deposits were hydroxyapatite and also indicated that they contained iron. Calcified tissue components included smooth-muscle cells, fibroblasts, and collagen fibers, as well as elastic fibers. No matrix vesicles or mitochondrial calcifications that may serve as nucleation sites for crystalline calcium phosphate were identified. Thus, the mechanism of calcification in IACI may be unique, and may relate to altered iron metabolism.

Occlusion of abdominal aorta in the newborn.

This is a report of a case of primary occlusion of the abdominal aorta in the first day of life that was managed operatively. Thromboembolic occlusion of the abdominal aorta is a surgical emergency. When promptly recognized and managed with arteriotomy and thromboembolectomy, morbidity and mortality should significantly decrease.

Oculo-auriculo-vertebral spectrum with vascular ring and other unusual anomalies.

An infant with the oculo-auriculo-vertebral (OAV) spectrum is described with the previously unreported association of rectal agenesis with recto-urethral fistula. The patient died at the age of 14 weeks from respiratory obstruction. Autopsy revealed the cause of death to be tracheal compression from a pulmonary artery sling. Previous reports of vascular rings within the OAV spectrum are reviewed, and it is concluded that these malformations are an uncommon, but clinically important, complication of the disorder.

Multiple coarctations of the pulmonary artery: scintigraphic appearance.

A case of angiography-proven multiple coarctations of the pulmonary arteries is presented. The patient had a history of long-standing exertional dyspnea and intranasal cocaine use. A chest radiograph suggested oligemia in the left lung and enlargement of the right pulmonary artery, prompting ventilation and perfusion radionuclide lung imaging. The combined scintigraphic and radiographic findings were indistinguishable from those caused by pulmonary emboli. Although rare, this entity should be included in the differential diagnosis of multiple unmatched perfusion defects on pulmonary ventilation-perfusion studies.

[Recurrent right-ventricular hypertrophy in patients with congenital isolated stenosis of the pulmonary artery and outlet tract of the right ventricle in the immediate and late periods after surgery]. / Obratnoe razvitie gipertrofii pravogo zheludochka u bol'nykh vrozhdennym izolirovannym stenozom legochnoi arterii i vykhodnogo trakta pravogo zheludochka v blizhaishie i otdalennye sroki posle operatsii.

The degree of hypertrophy of the right ventricle is proportionate to the size of the systolic pressure gradient between the right ventricle and pulmonary artery prior to operation. The involution of hypertrophy is dependent on the type of stenosis, age of the patient and the term of surgery.

Popliteal artery entrapment syndrome with thrombosed popliteal aneurysm: multidetector computed tomography angiography findings of a case.

Popliteal artery entrapment syndrome (PAES) is a rare congenital vascular pathology caused by the compression of the popliteal artery by adjacent muscle and tendinous structures. Popliteal artery aneurysm associated with this syndrome is extremely rare. A 45-year-old male suffering from pain at the right lower extremity during exercise was admitted to our hospital. Physical examination and Doppler imaging revealed a weak pulse at the posterior tibial artery and no pulse at dorsalis pedis and anterior tibial arteries. The patient was further evaluated with multidetector computed tomography angiography (MDCTA). MDCTA revealed PAES due to compression of the accessory fibers of the gastrocnemius muscle and related thrombosed popliteal aneurysm.

Myocardial ischemia secondary to congenital hypoplastic left coronary cusp in adult.

Coronary arterial obstruction associated with congenital aortic valve disease is rare in childhood, and has not been reported in adult. Here we reported a 49-year-old healthy woman with hypoplastic left coronary cusp resulting in myocardial ischemia in the territory of left main coronary artery.